Dystonia conference

Two weeks ago I went to a conference on cervical dystonia (hosted by the STDystonia group). My mom was my main motivator and being at the end of my first round of Botox I would not have had the guts to go without her. In fact, about a week before we went I was feeling so awful I called her to ask if we could bail and she talked me out of it. I'm so glad because I had such a good time, both hanging out with my mom (more on that later) and at the conference itself.

My lovely mom, who was my chaperone at the dystonia conference

Luckily, just two days before leaving I saw my new movement disorder specialist who gave me the prescription for my now-favorite med, klonopin, and I was feeling better already by the time we got on the plane - and increasingly improved throughout the conference. Although the klonopin much improved the super-painful muscle tension associated with dystonia, and eased the neck spasms quite a bit, I was still pretty symptomatic while I was at the conference with my head constantly leaning to one side or sometimes jerking (this happens especially when I talk). This forced me to interact with a  lot of people despite my obvious symptoms. With each interaction I got a little more brave (it helped that all of the people there either had the same problem or were a close relative or spouse of someone with the problem, so nobody reacted at all to it). In fact, in my bravest moment I even took the mic during the open discussion and made a few points to the whole group of 200+ people  - neck spasms and all. 

I learned a lot about dystonia, how to treat it to ease the symptoms, and how to deal with whatever symptoms remain after treatment. I met a LOT of really nice and caring people, both newly diagnosed and 'old hats' that were making a big effort to connect with people like me. It was a really informative and supportive experience.

But the real step forward came with my own accomplishments (and it helped that my mom was there to point them out to me). Even though the plane ride hurt like hell and my head was tilting weirdly as I walked through the airport and checked into the hotel and I could barely sit through all the talks, I survived. And it wasn't nearly as bad as I thought - I found ways to get through it and still get a lot out of it. I met people who were worse off and also getting through it and I realized even though I couldn't control where my head was all the time I could control a lot of other stuff that made it possible to travel and do all the things I did while I was there.

On the last day, my mom and I went into downtown Charleston SC (where the conference was held) to see the market and have a nice dinner together. We found the most amazing restaurant on the water and got wine and dessert and commended each other on how great we were both doing. It was one of those amazing moments you remember forever and I am so grateful to my mom for talking me into going (several times).

Painting the office

The last two weekends we did our first home project: we painted our office!!! It's a tiny room so was probably one of the easiest to paint (although there are nice cupboards and a window so we had to do a lot of taping for such a tiny room).

First we picked the color:

Then we bought a full can of paint (and the other stuff you need), donned 'paint clothes' and went crazy.

Layla actually ended up being a HUGE helper - we were really surprised that she could be so careful (sometimes more so than us!!). We all had to keep a close eye on each other, it's easy to drip!

After only an afternoon, we had finished the first coat and this morning in about an hour we touched it up with a quick second coat. And we are done (sorry I couldn't really get a good picture of the whole room - it is very small)!!

This was not only satisfying because it was our first home project (and it went well!) but also because  it was another milestone in my comeback after being stricken by dystonia - prior to my new meds and new botox this kind of project would have been WAAAY out of reach.

Maple Leaf Festival

Today we went to the Maple Leaf Festival to watch the parade and see all the craft vendors. It was wonderful weather and so much fun to get out and enjoy it. Even though I am only 1 week into my second Botox treatment my neck held up really well and I made it through the whole event and a bunch of other family activities before and after. But I did lean my head on my husband a few times for rest and one of these times our friend snapped this quick picture (thinking I was being affectionately cute). Here is an example of where I am learning how to hide my condition - although it could easily be hidden because it is not freaking me out as much anymore but mostly because the symptoms are much controlled by the so-wonderful Botox and klonopin (mostly Botox).

The highlight of the festival was a house completely decked out for Halloween. They had a very real-looking guillotine on the curb (which had a line of people waiting to take selfies with it), and a yard full of super-spooky halloween decorations - heads hanging from a clothesline, skeletons climbing up the house, a shivering mummy, a jumping spider, corpses everywhere in all kinds of situations and all kinds of other stuff. There was a huge crowd of people just standing at the fence looking at all of it.

In both pictures you can see a little red clown wandering around. The clown was hanging around looking spooky but also handed out candy every once in a while. Layla was lucky enough to get a sucker. Then she watched closely when the clown eventually disappeared back into the house. And later, when we were buying a craft I heard her tell someone, "I got this sucker from the spooky clown at the Halloween House. Then he went inside to watch TV and have some lunch." 

A closer shot of some of the spookiness of the Halloween House:

Cervical Dystonia - my story

In February 2014, my neck started twitching a little bit. I felt it just before I went to bed, some nights worse than others. It lasted about 3 weeks. Then on a road trip to visit family in Iowa, it started twitching during the day and ached a little on the trip. I started complaining about it, took some pain meds and found myself resting my head on my hand as much as I could. The day after we returned from our trip, I found I suddenly could not hold my head straight forward at all. I took the day off work, went in to the chiropractor (who was baffled, but still assured me it was just a pulled muscle), then saw a massage therapist (who was also baffled, but agreed with the chiropractor that it must be a pulled muscle), and feeling no better the next day, I returned to work and just held my head in my hands the whole day. It was tiring, to say the least. As the week went on, and turned into the next week and the next, I slowed down more and more and began to cancel things and cut back. Also I was finding that my neck was starting to spasm and jerk and my hands couldn't even hold my head straight (it would spasm right out of my grasp!). I eventually sprained my wrist, scraped up my elbows bracing them on my desk, and pinched a nerve in my back. I saw my general doctor, who was also baffled and prescribed a high dose of valium, to take daily until the 'problem' resolved. This helped with the pinched nerve, but made me feel awful. I started seeing a physical therapist, another massage therapist, and an osteopath who did craniosacral therapy. They were all baffled, and also desperate to try to help. In their desperate 'helping,' they mostly made it worse, with the exception of the craniosacral therapy (this is a very gentle approach to body work), which really helped - it helped with the pinched nerve and provided a little relief so that I could keep working a little bit, although it was only a bare minimum. Also one of the massage therapists, in a brilliant moment of guessing at what might help, showed me how by sitting still and focusing my attention AWAY from the muscles that I was fighting, I could actually control it just a tiny bit - this was the beginning of learning how to live with this although I didn't realize it yet.

I finally had enough with my chiropractor, who after 4 weeks was still singing the same song about it being a pulled muscle. At home we were having our deck stained, and the person we hired to do it took one look at me and asked if he could pray for me - then he got down on his knees right there and prayed. I must have looked pretty bad. I asked him if he knew of a good chiropractor and he gave me a detailed review of all of the ones in town, with his favorite just down the street. I went straight over to her and that's when I finally got a diagnosis.

The new chiropractor told me, as gently as she could, that there was a neurological disorder called cervical dystonia that caused all the symptoms that I had and that I should see a neurologist as soon as possible to check if this was what I had. I was still thinking it all stemmed from a pulled muscle or pinched nerve somewhere, and was devastated to hear there was this other, much more horrible sounding thing that it could be (and when I looked it up, it matched all my symptoms... oh what an awful night of frenzied internet-searching that was). 

I went to the neurologist, who agreed with the chiropractor that it might be a dystonia, but told me not to lose hope - dystonias are treatable with Botox (usually), and besides, we still weren't sure that it wasn't a pinched nerve or pulled muscle. He wanted me to wait another month, get an MRI, and see what happened with my symptoms. He encouraged me to keep taking the valium, even though it was still making me feel awful.

So for a month I hoped that it wasn't a dystonia. On days when I felt better I would cheer and be happy and when my symptoms were worse I despaired. The therapists I was seeing (the osteopath, massage therapists, and physical therapist) who knew nothing about dystonia all assured me that I couldn't possibly have dystonia and kept my hope alive that it was all going to go away. Even after the MRI came back suggesting a dystonia (the conclusion they make when there is no evidence of a pinched nerve or any other clear problem that might cause my symptoms). But MRIs are limited and I continued to hope it was all going to go away, even as I went back to the neurologist and he diagnosed me with cervical dystonia and started the process to get the Botox approved. And to add to my own denial, I kept having stretches that were better than others, and after a few months (it took almost 3-4 months from my first neurology appointment to the actual appointment for my first injections), in little bits, I was starting to feel like I was getting better - or at least, I was learning how to better tolerate the condition. I could hold my head straight on my own sometimes, the spasms weren't wrenching my body so painfully, and I was able to decrease the valium a bit (not a small task, if you know much about benzo withdrawal). So for that first month after my diagnosis I tried hard to believe I wouldn't need Botox after all because somehow I was going to get better on my own.

But between the good days were bad days, and some days were really bad days. Sometimes I had to spend the whole day or even weeks on the floor of my living room because the combination of the neck spasms themselves and the trauma they were causing to my body was just too much for me to even get up. And I couldn't predict when I would feel ok and when I would be so contorted by the pain and neck spasms that I could hardly drive or walk, so I couldn't commit to doing anything that involved leaving my house. Although staying home drove me crazy, and eventually I ventured out anyway. I figured out that I could survive for short trips to a few places like the park or the pool as long as I could find a way to lie down a lot and get over my own self-consciousness over my crazy-looking head (although I found that if I didn't interact with anyone, most people wouldn't notice). But it was still miserable - I could go out, but it exhausted me and I couldn't do much, and the rest of the time I was on the floor, trying not to think about how much my neck hurt. As the date of my Botox injections drew nearer I switched my attitude from one of hope that I wouldn't need Botox to one of hope that Botox would give me some kind of relief from the misery I was in. This change in attitude marked the end of my denial.

So I took action. Just before getting my first Botox injections, I finally made contact with some people who had cervical dystonia. The first person I met with cervical dystonia, MT, drove right over after we found each other, gave me a hug, sat with me at my kitchen table, and told me not to be afraid of Botox or of cervical dystonia. She had been treated successfully with Botox for 17 years and talked me through what it would be like and how much it would probably help me. She showed me how her neck had been and how much better she was now, definitely not stuck on her living room floor most of the time. Most importantly, she had a matter-of-fact attitude about it that really changed my perspective. Up until then, this had been a weird thing I had never heard of and for all I knew I caused it to myself. It seemed scary and foreign. MT helped me realize that it's nothing more than a medical condition and as far as medical conditions go it's not too bad of one because it's treatable. Traumatic, possibly difficult to manage and in some cases debilitating, but treatable. And in MT's case, at least, she had managed to treat it quite well.

In the middle of all this I met another amazing woman, Ms. Z, who had had generalized dystonia. Ms. Z told me her story about how she had spasms all over her body, night and day, and hardly functioned. Botox is not helpful for generalized dystonia as there are too many muscles involved. Like many of us who have dystonia, Ms. Z was overcome by fear and panic as her symptoms took away her life and career and made her feel miserable. So with great compassion for the situation I was currently dealing with, she encouraged me to learn to accept my life as it is, so that I could live as much as possible without fear. This would help me feel better (because you always feel better when you are happy and relaxed vs. fearful and tense) and would also be critical for me to think clearly to find the right treatment and path toward recovery (because calm leads to clarity whereas blind panic leads nowhere). She reminded me that even though I felt like I was about to lose my job and my life (as she had felt) - this wasn't the case at all. She pointed out all the things I could still do (for example, talk, see, feed myself, think clearly, etc - coming from someone with generalized dystonia this was a profound realization). I could do all those things, and I was still working a little bit, as much as I could, and had gotten nothing but support from my colleagues and boss. I didn't know if I would get better, but I didn't know that I wouldn't get better either. Or (and this came directly from Ms. Z, who was full of hope) - I also didn't know where my job could go if I didn't get better but still didn't quit - sometimes there's a path that you never knew existed. By the way, after 5 years of generalized dystonia Ms. Z's symptoms dramatically improved and she is one of the lucky ones that has gone into remission.

So, as I was slowly coming to accept that I have cervical dystonia and my life-path may never be the same as I had originally planned it, I went in for my first Botox injections. And they helped, about 70%. I could keep my head straight. I could almost have a conversation with someone without them noticing there was something off about my neck. I had enough energy to work all day (most days), interact with my family, even go out to eat - sometimes all in the same day! It wasn't perfect though - my neck still bothered me, and I had to work really hard to not think about it. People did notice it, as it was still stiff and sometimes obviously bothering me. Certain things were still impossible, like driving long distances and sitting through more than an hour of meetings. But I was very, very grateful to be about 70% back to my normal life.

After my first Botox treatment - doing much better but my right should drooped a little and I still struggled a little to keep my head straight

And then i realized that what MT and Ms. Z had told me was true - this was treatable, and staying calm about it would lead to the best possible outcome. I had already discovered and had great success with Botox - but what else could I do? I sought out several different physical therapy programs designed for people with cervical dystonia and through these I started working harder on strengthening my back, improving my posture, and doing the things that generally make you feel better - and this helped. Then on the advice of other people I had met with cervical dystonia I switched doctors, to a movement disorder specialist who specifically treats people with dystonia and is very experienced with giving Botox into the neck muscles. The new doctor not only improved the effectiveness of my Botox treatments, but he also talked me into trying klonopin, a muscle relaxant related to valium that actually helped my symptoms without the awful effects I had with valium. With klonopin I actually had a back-up plan for those times when the Botox didn't quite cut it - and this was also a huge, huge step forward since Botox doesn't always last the whole duration between treatments (treatments must be spaced at least 12 weeks apart to reduce the chance of becoming immune to it and having it no longer be effective).

After my second Botox treatment with a Movement Disorder Specialist, feeling (and looking) even better!

I am happy to say I am now back at work and, although still cautious, I am not too far off track from where I want to be (although I have changed my expectations somewhat, and am much more accepting of wherever I end up). I am also feeling stronger and healthier, and importantly learning how not to let my neck bother me as much, both when it 'feels funny' and also when it might look funny to other people. I'm finding that if I don't pay attention to it other people don't either. 

And through all this there were some positives. My relationship with my family has grown much, much stronger. My mom, who was my constant support through all of this, has truly become my best friend. I am in awe of the amount of energy she was able to put out when I needed it and am so grateful for all the fun we had together when I started to feel better - and even when I didn't. She helped me learn how to live and be happy no matter how much emotional or physical pain I'm in. My husband was my hero through this and proved he can move mountains to keep the ship from going down, and he still give me a neck massage at the end of the day. And my daughter, who was always a momma's girl, showed her support by giving me the space I needed when I needed it the most, which took a lot of growing up and I'm so proud of her for this.

I am also forever changed in my perspective of other people who have to face challenges. I have an aunt who is blind who I have always looked up to for her amazingly functional can-do attitude, and I thought of her often on this journey. This experience has made me more aware and compassionate toward all the people, like her, who have had to figure out a way around something that most people take for granted or never think about. In a very short period of time I went from healthy and normal to disabled and now I will never forget to enjoy every minute to the most that I can.

Cervical Dystonia

In March, I started having symptoms of what turned out to be a neurological movement disorder called cervical dystonia.

What is cervical dystonia? (I wondered this too)

Dystonias are involuntary muscle spasms. Your brain sends the wrong message to a muscle telling it to contract when you don't really want it to. These can occur in all kinds of different ways. For example writer's cramp, golfer's 'yips' and dysphonia (muscle spasms of the vocal chords) are all dystonias. Eye twitches are also dystonias. You can have a generalized dystonia, where spasms happen all over the body, or a focal dystonia that only occurs in one area (like eye twitch). The most common type of dystonia is of the neck (called cervical dystonia). This is the type that I have.

Scientists really don't understand what causes dystonia. There is a type of generalized dystonia that children get that has been studied better than most, and all they really know about that one is the gene that is involved. In other types of dystonias, it seems there is a problem with neurotransmitters that are important for motor control (e.g. dopamine). But, many different genetic mutations can lead to dystonia and only about 25% of these are even known - so there is still a lot that we don't know. What does seem clear is that in people with dystonia, there is a disconnection between the brain and the nerves in the muscles that the brain is trying to control. What is really weird about dystonia is that in some patients the symptoms can go away (about 10-20%), and the patient can go into remission - but most patients that go into remission will eventually 'relapse' and have the dystonia reoccur (and not always in the same place or same way). This suggests that sometimes the brain or body can find a way around the issue that causes the dystonia, at least temporarily. Dystonias are not typically progressive (although they have been known to 'spread' from one site to another in some patients) and have no effect on cognitive function or lifespan.

Even though it is the third most common movement disorder (behind Parkinson's and essential tremor), almost no one has heard of dystonia. This is too bad because that means it often gets misdiagnosed, and misdiagnosing a dystonia can lead to a LOT of unnecessary emotional and physical trauma. People have been told they're crazy or lazy, they have had surgery that actually made the problems worse, they have spent thousands upon thousands of dollars seeking out medical help from the wrong specialists (yes, even doctors often don't recognize dystonias!), and on and on. The average person with dystonia goes to 7 doctors which takes over a year before they get the correct diagnosis. I had to go to only 4, and was diagnosed in about 2 months.

Like many chronic illnesses, dystonias can manifest in as many ways as people that have them. Cervical dystonia is no exception. Cervical dystonia is when muscles in your neck contract and your head is pulled to one side, up or down, or some combination of these. Every person is different in the direction their head is turned and how their symptoms appear. It can be a constant pull or a pulling-jerk or happen with or without a tremor. Some people have just a tremor and no pull. It can also be a VERY obvious deviation of the head (or tremor, or both) or it can be totally hidden (just something the patient feels). It can be off and on, or constant. Mine is a constant pull with some jerking (no tremor), with a deviation of the head that is 10 degree tilted to the left (ear to shoulder), 10 degree turned to the right and a moderate 'retrocollis' (looking up). In the next post I'll tell the story of how my symptoms started, my diagnosis and treatment so far.

The muscle contractions caused by dystonias can be very, very severe. Even if the symptoms aren't visible to others, it can cause an immense amount of pain and suffering. It can be exhausting, horrifically damaging to the body (after just 3 months untreated, permanent damage can occur to the spine), causing pinched nerves, broken teeth and bones, herniated discs, misalignments of the jaw and asymmetries of the face and body. Below is a before-dystonia and after-dystonia (before treatment) picture of a person named Rogers Hartmann who developed cervical dystonia in her 30s (like me) which caused her head to pull so hard her body was bent completely over. Rogers, like many of us dystonias, lost a lot of weight after her symptoms started - this is common due to all the calories burned by the muscles spasms (and difficulty with eating). When untreated, I do not look quite this bad, but my body does get pulled something like the picture shown. As you can imagine, it makes it really difficult and tiring to do simple tasks like getting a drink of water, brushing your hair and showering.

Dystonias are treatable but not curable. Focal dystonias can be treated with Botox, which temporarily blocks the nerve from sending constant signals to the spasming muscles. However Botox treatment can be tricky because the dose and location of injections must be optimized - it takes an experienced doctor to get it right and often several attempts before it's really effective. And some dystonias are better treated with Botox than others. There are some oral medications that help, but these often have side effects and it can take a while to find the best one for each patient. Other options are denervation surgery, where the nerves are cut (this is permanent, but when it helps it can be really helpful). The most recent, and maybe most promising treatment is deep brain stimulation, where electrodes are put into the brain through surgery and send pulses to the parts of the brain that are malfunctioning (using a pace-maker type device). It can take up to a year to optimize the frequency and strength of the pulses to minimize symptoms, and there are still some issues with the surgery itself and maintenance of the system, but it does often help especially in those cases where Botox and other methods fail. What we really need to improve treatment is a better understanding of the cause so that we could target and 'fix' the root of the problem, but unfortunately we are still a long ways from this. But the past 20 years has been revolutionary in terms of treatment options and there is a lot of hope that we will continue to improve those treatments and develop new ones, and find the right thing for each patient faster so all dystonians have a better chance at a good quality life. So if you are reading this and you have cervical dystonia or any kind of dystonia please stay positive and keep working to find something that helps you, because there are a lot of things you can try and a lot of resources on how to be an advocate for yourself to find the best outcome.

Almost a year in Kansas

As we approach our 1-yr anniversary of moving to Kansas, it still seems so strange to be in here instead of Seattle. Maybe that's partly because we still haven't hung pictures or bought furniture (there's a reason for that, which I'll post about next), but mostly it's because it was such a big change. Every season is so different, every routine still so new, having family nearby offers so many unexpected opportunities (which has been so great!), we're still in the 'new-friend' process and getting used to our new jobs, and finding new things we like in the new city we live. It's been mostly fun and a good experience, we have been reminded of how much we love the midwest, the weather, the people, the culture, etc. But we still often talk about how much we miss Seattle!!

To help ease the transition, we bought cookbooks for two of our favorite places in Seattle, Trophy Cupcakes and Molly Moon's Ice Cream (you can get both on Amazon). Delicious!